Authors:

MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK.

Objective: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare multisystem disease associated with a plasma-cell dyscrasia. Although pachymeningeal involvement has occasionally been described, MRI of the central nervous system (CNS) has not yet been extensively investigated.

Methods: We retrospectively evaluated CNS MRI in Europe's largest single-center cohort of POEMS syndrome. Read More

The POEMS syndrome is a rare and multisystemic disease characterized by the presence of polyneuropathy, organomegaly, endocrinopathy, presence of M protein, and alterations in the skin. The existence of bone lesions is frequent, being generally blastic or mixed and rarely lytic. We present the case of a 54-year-old male patient diagnosed with POEMS syndrome, with atypical presence of lytic lesions, who was referred for an 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET-CT) study for initial staging of the process. Read More

Glomeruloid hemangiomas were first described by Chan in 1990 as a cutaneous marker of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal proteinemia, and Skin changes). POEMS syndrome is a multisystem disease with a significant cutaneous involvement. Among its manifestations, hemangiomas are common, observed in up to one third of the patients, and, although specific, the glomeruloid subtype is very rare. Read More

POEMS syndrome is a rare paraneoplastic disease associated with monoclonal plasma cells; however, the pathogenic importance of plasma cells remains unclear. We performed comprehensive genetic analyses of plasma cells in 20 patients with POEMS syndrome. Whole exome sequencing was performed in 11 cases and found a total of 308 somatic mutations in 285 genes. Read More

Authors:

The histology of skin lesions of TAFRO (thrombocytopenia, anasarca, reticulin fibrosis/renal failure, and organomegaly) syndrome has rarely been reported. We report herein two cases of TAFRO syndrome with characteristic vascular skin lesions. The lesions resembled a tufted angioma, although those of case 1 partially resembled a glomeruloid hemangioma, which was known as a specific lesion in POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes), a variant of multicentric Castleman disease (MCD). Read More

The diagnosis of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome requires polyneuropathy and monoclonal plasma cell proliferation as two mandatory criteria. Our aim was to summarize clinical manifestations and treatment responses of POEMS variants with no evidence of monoclonal gammopathy. We queried all medical documentation of patients referred to Peking Union Medical College Hospital from August 2012 to July 2017, and reviewed the clinical and laboratory features of 13 patients with atypical POEMS syndrome with undetectable monoclonal gammopathy, and compared to prototypes published. Read More

A 65-year-old woman was referred to our department with recurrent pericardial effusion. Her serum vascular endothelial growth factor was high, serum M-protein was positive, and nerve conduction velocity of extremities was decreased. Therefore, she was diagnosed with Crow-Fukase (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes:POEMS) syndrome, which is characterized by the presence of plasma cell dyscrasia with them. Read More

Glomeruloid haemangioma (GH) is considered a specific marker of POEMS syndrome, despite some published GH cases unrelated to POEMS syndrome. To present two cases with GH and atypical presentations of Erdheim-Chester disease (ECD) or POEMS syndrome, as well as a retrospective monocentric study of histologically-confirmed GH. Clinical, biological and histological data of the patients is presented. Read More

Authors:

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. Read More

Hypothyroidism is the most common endocrine disorder in POEMS syndrome. In this single-center prospective study, 36 patients with newly diagnosed POEMS syndrome were treated with lenalidomide (10 mg daily for 21 days) and dexamethasone (40mg once per week) for 12 cycles. Hypothyroidism was demonstrated in 58. Read More

Background: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome still has no standard treatment. On the basis that both POEMS syndrome and myeloma have an underlying plasma cell dyscrasia, anti-myeloma therapy can be expected to be useful for POEMS syndrome. Chimeric antigen receptor T (CAR-T) cells targeting B cell maturation antigen (BCMA) has been used in the treatment of relapsed and refractory multiple myeloma (RRMM). Read More

POEMS syndrome (acronym consisting of: polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is an uncommon disorder associated with an underlying plasma cell dyscrasia. There is no single specific test for POEMS, and due to its rarity and heterogeneity, patients are often mis- or underdiagnosed. Castleman disease (CD) is a rare lymphoproliferative disorder, closely related to POEMS syndrome; ~11%-30% of POEMS patients are associated with concomitant CD. Read More

Authors:

Background: POEMS syndrome is a rare neoplastic syndrome reflected by plasma cell disorder. It is composed by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. It is also reported to associate with Castleman disease. Read More

POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. Treatment may be either local or systemic and is aimed at the monoclonal plasma cell disorder. Read More

The finding of an IgM monoclonal gammopathy often represents a diagnostic challenge. In fact, there are many pathological disorders associated with this condition, each of which has distinctive characteristics and requires specific clinical, instrumental, and laboratory assessments to set the appropriate treatment. This review has two aims. Read More

Objective: To investigate the sensitivity and the specificity of serum vascular endothelial growth factor (sVEGF) for the diagnosis of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome in patients with a neuropathy (NP) and to identify confounding causes of raised vascular endothelial growth factor (VEGF) in this context to improve accuracy.

Methods: We studied the specificity and sensitivity of sVEGF for the diagnosis of POEMS syndrome in a cohort of 195 consecutive patients with an NP in serum samples from June 2009 to November 2013, including 27 untreated patients with POEMS syndrome. We then studied VEGF in other neuropathies and analyzed causes of elevated VEGF in a multiple logistic regression analysis in a larger cohort of 236 patients including 168 with a non-POEMS NP and 68 without NP. Read More

Introduction: Lenalidomide is an effective therapy of POEMS syndrome. However, there is concern that exposure to lenalidomide may reduce the efficiency of blood cell collection in persons who may eventually receive an autotransplant. We studied the impact of lenalidomide therapy on subsequent blood cell mobilization and collection including frequency of blood CD34+ cells and CXCR4 expression before and after mobilization with cyclophosphamide and granulocyte-colony stimulating factor (G-CSF). Read More

Recent Findings: With the development of large multicentre national cohorts of patients, POEMS syndrome is evolving into a well characterized multisystem hematoneurological syndrome. Without early diagnosis significant disability results from the neuropathy. Read More

POEMS syndrome is a rare multiple organ involvement of the parasympathetic syndrome associated with abnormal plasma cells, mostly with high-dose chemotherapy and stem cell transplantation for the treatment. Recently, more treatment attempts to treat POEMS syndrome have been utilized so as to improve the efficacy and safety for the patients with POEMS syndrome, such as immunomodulator, alkylating agent, cytokine-induced killer cells and so on. Lenalidomide has a significant effect on relapse/refractory POEMS syndrome and patients with endocrinopathy. Read More

Authors:

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare systemic disease, often unrecognised in the primary care setting. POEMS syndrome is associated with plasma cell dyscrasias and upregulation of vascular endothelial growth factor leading to systemic oedema, papilloedema and pulmonary hypertension. A wide constellation of presenting symptoms often leads to late diagnosis. Read More

Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.

The introduction of novel agents in the management of multiple myeloma and related plasma cell dyscrasias has changed our treatment approaches and subsequently the outcome of patients. Due to current advances, the European Myeloma Network updated the diagnostic and therapeutic recommendations for patients with Waldenström's macroglobulinemia (WM), AL-amyloidosis, monoclonal immunoglobulin deposition disease (MIDD), POEMS syndrome, and primary plasma cell leukemia. For patients with WM, the combination of rituximab with chemotherapy remains the treatment cornerstone, while the Bruton-tyrosine kinase inhibitor ibrutinib has been introduced and approved for relapsed/refractory disease. Read More

Castleman's disease (CD) is a rare benign lymphoproliferative disorder. We are presenting three cases of CD of which one is unicentric CD, and the other two are an idiopathic multicentric CD. One of the two multicentric cases is associated with POEMS syndrome. Read More

Methods: From a cohort of 194 patients with FAP, 13 of 84 patients (15%) of French ancestry had late-onset demyelinating TTR-FAP. We compared clinical presentation and electrophysiology to a cohort with CIDP and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome. Read More

Authors:

Department of Hematology and Immunology, Kagoshima University Hospital.

POEMS syndrome is often complicated by pulmonary hypertension. The standard therapy for patients with POEMS syndrome is high-dose chemotherapy followed by autologous stem cell transplantation. However, the safety of high-dose chemotherapy for patients complicated with pulmonary hypertension remains unclear, and the optimal therapy for these patients is yet to be establishment. Read More

Although hematopoietic stem cell transplantation (HSCT) may increase the curability of refractory hematologic diseases, it requires complication management due to a long-term immunocompromised state. We experienced a case who received an autologous peripheral blood stem cell transplantation (Auto-PBSCT) for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) and developed cutaneous Mycobacterium chelonae infection. It is clear that attention needs to be paid to prevent bacterial, fungal and viral infection after HSCT. Read More

Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. Read More

Authors:

Department of Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo 113-0033, Japan. Electronic address:

Glomeruloid hemangioma is a rare cutaneous lesion that has been considered as a specific cutaneous marker of POEMS syndrome. Herein, we present the first case of glomeruloid hemangioma associated with TAFRO syndrome, a unique variant of idiopathic multicentric Castleman disease. The patient is a 74-year-old woman presented with fever, cervical lymphadenopathy, thrombocytopenia, bilateral pleural effusions and ascites. Read More

Lenalidomide, a novel immunomodulatory agent, is a kind of thalidomide derivatives, which shows a good efficacy and safety for hematological system diseases. This review is aimed to evaluate the efficacy and safety of lenalidomide in treatment of patients with multiple myeloma, chronic lymphocytic leukemia, acute myeloid leukemia, non-Hodgkin's lymphoma, classical Hodgkin's lymphoma and POEMS syndrome at their replased or refractory state. At the same time, this review focuses on the newest clinical research and the latest application progress of lenalidomide for relapsed or refractory hematological system diseases. Read More

Flushing is the subjective sensation of warmth accompanied by visible cutaneous erythema occurring throughout the body with a predilection for the face, neck, pinnae, and upper trunk where the skin is thinnest and cutaneous vessels are superficially located and in greatest numbers. Flushing can be present in either a wet or dry form depending upon whether neural-mediated mechanisms are involved. Activation of the sympathetic nervous system results in wet flushing, accompanied by diaphoresis, due to concomitant stimulation of eccrine sweat glands. Read More

Autologous stem cell transplantation (ASCT) is considered as standard of care in patients with multiple myeloma (MM) patients aged 65 years or younger. We analyzed data of 94 patients of plasma cell dyscrasias who underwent 95 autologous transplants at our institute from October 2003 to Aug 2016. Other than 76 patients of newly diagnosed multiple myeloma, we also transplanted two patients of POEMS syndrome, two patients of plasma cell leukemia, three patients of concurrent light chain deposition disease, three patients of multifocal plasmacytomas, and eight patients of isolated light chain myeloma. Read More

Authors:

Comparing results from jitter studies performed with voluntary and electrical activation is difficult to perform quantitatively, particularly in complex signals as seen in reinnervation. High jitter values in individual spikes in these multispike signals can be missed with both activation methods, which introduces a bias towards more normal values.With voluntary activation, triggering on a spike from an abnormal end-plate in multispike potentials will overestimate individual jitter values and the number of abnormal jitter values. Read More

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